“…The resulting whorled pattern of corneal abnormality in heterozygous females is presumably a consequence of heterogeneity of expression in limbal stem cell clones (functional mosaicism for ␣-galactosidase expression), similar to the phenomenon described in this study. In reported cases of vortex keratopathy, the whorls always turn clockwise, and it has been suggested that this finding is in response to electrical fields across the corneal surface (Dua et al, 1993(Dua et al, , 1996. In contrast, in our model, both clockwise and counterclockwise whorls were observed, apparently randomly with respect to left and right eyes, but only in the very centre of the cornea.…”